Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children (Q33380064)

26 July 2017

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title

Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children (English)

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thrombotic thrombocytopenic purpura

26 July 2017

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5

Agnès Veyradier

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author name string

Chantal Loirat

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26 July 2017

Jean-Pierre Girma

2

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26 July 2017

Céline Desconclois

3

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26 July 2017

Paul Coppo

4

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26 July 2017

Agnès Veyradier

5

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26 July 2017

24 June 2008

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26 July 2017

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26 July 2017

24

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26 July 2017

1

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26 July 2017

19-29

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https://scigraph.springernature.com/pub.10.1007/s00467-008-0863-5

26 July 2017

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cites work

Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura

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Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome

21 January 2018

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Addition of rituximab to standard therapy improves response rate and progression-free survival in relapsed or refractory thrombotic thrombocytopenic purpura and autoimmune haemolytic anaemia.

21 January 2018

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12 December 2020

Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome

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12 December 2020

Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura

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12 December 2020

Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome

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12 December 2020

Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen

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12 December 2020

Thrombotic thrombocytopenic purpura attributable to von Willebrand factor-cleaving protease inhibitor in an 8-year-old boy.

1 reference

12 December 2020

Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).

1 reference

12 December 2020

ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases

1 reference

12 December 2020

Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura

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12 December 2020

Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura

1 reference

12 December 2020

von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.

1 reference

12 December 2020

Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity

1 reference

12 December 2020

Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease

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12 December 2020

Rituximab therapy for thrombotic thrombocytopenic purpura: a proposed study of the Transfusion Medicine/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune-mediated disorders

1 reference

12 December 2020

Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura

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12 December 2020

Desmopressin, an unexpected link between nocturnal enuresis and inherited thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).

1 reference

12 December 2020

Thrombotic thrombocytopenic purpura and its diagnosis

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12 December 2020

Acquired idiopathic thrombotic thrombocytopenic purpura: arguments for an autoimmune disease

1 reference

12 December 2020

The activity of the von Willebrand factor cleaving protease ADAMTS-13 in newborn infants

1 reference

12 December 2020

Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases

1 reference

12 December 2020

Role of thrombospondin-1 in control of von Willebrand factor multimer size in mice.

1 reference

12 December 2020

Evaluation and management of patients with thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura.

1 reference

12 December 2020

An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers

1 reference

12 December 2020

Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity

1 reference

12 December 2020

In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura

1 reference

12 December 2020

ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.

1 reference

12 December 2020

Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in children and adolescents

1 reference

12 December 2020

ADAMTS 13 genotype and vWF protease activity in an Italian family with TTP.

1 reference

12 December 2020

Thrombotic Microangiopathies

1 reference

12 December 2020

Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement

1 reference

12 December 2020

Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).

1 reference

12 December 2020

Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement

1 reference

12 December 2020

Assays of ADAMTS-13 activity

1 reference

12 December 2020

Studies on thrombopoiesis. I. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency

1 reference

12 December 2020

Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation

1 reference

12 December 2020

Thrombotic thrombocytopenic purpura associated with von Willebrand factor-cleaving protease (ADAMTS13) deficiency in children

1 reference

12 December 2020

Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity

1 reference

12 December 2020

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry: a community perspective of patients with clinically diagnosed TTP-HUS.

1 reference

12 December 2020

ADAMTS13 gene mutation in congenital thrombotic thrombocytopenic purpura with previously reported normal VWF cleaving protease activity

1 reference

12 December 2020

Management of thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Rituximab for acute plasma-refractory thrombotic thrombocytopenic purpura. A case report and concise review of the literature

1 reference

12 December 2020

Plasma therapy in thrombotic thrombocytopenic purpura: review of the literature and the Bern experience in a subgroup of patients with severe acquired ADAMTS-13 deficiency

1 reference

12 December 2020

Complement regulatory genes and hemolytic uremic syndromes

1 reference

12 December 2020

Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency

1 reference

12 December 2020

Thrombotic thrombocytopenic purpura as a rare complication in childhood systemic lupus erythematosus: case report and literature review

1 reference

12 December 2020

Plasma levels of von Willebrand factor regulate ADAMTS-13, its major cleaving protease

1 reference

12 December 2020

Comparison and stability of ADAMTS13 activity in therapeutic plasma products

1 reference

12 December 2020

Changes in von Willebrand factor-cleaving protease (ADAMTS13) activity after infusion of desmopressin

1 reference

12 December 2020

Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion.

1 reference

12 December 2020

Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome

1 reference

12 December 2020

FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay

1 reference

12 December 2020

Paediatric case report of an acquired autoimmune thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure

1 reference

12 December 2020

Thrombotic thrombocytopenic purpura: a moving target

1 reference

12 December 2020

The molecular biology of thrombotic microangiopathy

1 reference

12 December 2020

Genetic defects leading to hereditary thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Plasma therapy in von Willebrand factor protease deficiency

1 reference

12 December 2020

ADAMTS-13 levels in fresh, stored, and solvent detergent treated plasma

1 reference

12 December 2020

Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Pathology of thrombotic thrombocytopenic purpura in the placenta, with emphasis on the snowman sign

1 reference

12 December 2020

Recovery and half-life of von Willebrand factor-cleaving protease after plasma therapy in patients with thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice

1 reference

12 December 2020

Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family

1 reference

12 December 2020

Successful treatment of a young infant who developed high-titer inhibitors against VWF-cleaving protease (ADAMTS-13): important discrimination from Upshaw-Schulman syndrome

1 reference

12 December 2020

Factor VIII concentrate-responsive thrombocytopenia, hemolytic anemia, and nephropathy. Evidence that factor VIII:von Willebrand factor is involved in its pathogenesis

1 reference

12 December 2020

Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H.

1 reference

12 December 2020

Hemolytic Uremic Syndrome

1 reference

12 December 2020

Molecular characterization of four ADAMTS13 mutations responsible for congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).

1 reference

12 December 2020

Recurrent idiopathic thrombotic thrombocytopenic purpura: a role for vaccination in disease relapse?

1 reference

12 December 2020

Dissociation between the level of von Willebrand factor-cleaving protease activity and disease in a patient with congenital thrombotic thrombocytopenic purpura

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12 December 2020

von Willebrand factor: two sides of a coin

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12 December 2020

Severe ADAMTS-13 deficiency in childhood

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12 December 2020

von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome

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12 December 2020

Deficient activity of von Willebrand factor-cleaving protease in patients with Upshaw-Schulman syndrome

1 reference

12 December 2020

Normal levels of ADAMTS13 and factor H are present in the pharmaceutically licensed plasma for transfusion (Octaplas) and in the universally applicable plasma (Uniplas) in development

1 reference

12 December 2020

Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia

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12 December 2020

Pathogenesis of thrombotic microangiopathies

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12 December 2020

ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura

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12 December 2020

Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis.

1 reference

12 December 2020

VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13

1 reference

12 December 2020

Case series of thrombotic thrombocytopenic purpura in children and adolescents

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12 December 2020

Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma

1 reference

12 December 2020

Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity.

1 reference

12 December 2020

10.1007/S00467-008-0863-5

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DOI

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26 July 2017

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